The clinical profile of pseudomyxoma peritonei is normally increasing abdominal circumference and confirmation of mucous in the abdominal cavity (jelly belly). But, disease history including appendicitis or mucous as coincidental finding are alternative forms of presentation.
|Patient with advanced pseudomyxoma peritonei. Click to enlarge.
Distribution of mucous in the abdominal cavity is characteristic. Mucous and attached tumor are usually observed:
- in the greater omentum
- under the right diaphragm
- in the paracolic gutters bilaterally
- around the liver and gallbladder
- in the abdominal cavity
With advanced disease, tumor is also seen under the left diaphragm, omental bursa and on the small intestine.
In mildly malignant tumors the characteristic symptoms are :
- growth of the abdomen with uncharacteristic bloating or pain. In extreme cases, pressure in the diaphragm can cause incapacitating heavy breathing, and susceptibilty for complications such as aspiration pneumonia and deep vein thrombosis/lung embolism.
- polyuria from pressure on the bladde.
- iileus due to compression of segment of the intestines.
- pain resembling the symptoms of appendicitis.
- hernia with tumor due to intra-abdominal pressure caused by mucous volum.
- metastases to the ovaries (often diagnosed as primary ovarian cancer).
Few, slightly atypical epithelial cells are often observed in the mucous, and often both ovaries are involved. It was previously thought that tumor originated from the ovaries, but immunohistochemical examinations indicate that tumor in the ovaries is due to metastasis. For this reasjon tumor is often misinterpreted as low-grade ovarian cancer. The appendix is usually the origin, but other primary localizations have also been reported.
In women with peritoneal metastases, the ovaries are commonly affected. There may be significant tumor growth inside the ovaries or on the surface. For known colorectal cancer, there is more than 50% chance that an ovarian mass is a peritoneal metastasis.(6)
More malignant tumors have a tendency to debut with specific organ symptoms due to infiltration.